Fetal/neonatal alloimmune thrombocytopenia (FNAIT) is a relatively rare condition (1/1000–1/2000) that was granted orphan status by the European Medicines Agency in 2011. Clinical consequences of FNAIT, however, may be severe. A thrombocytopenic fetus or new-born is at risk of intracranial hemorrhage that may result in lifelong disability or death. Preventing such bleeding is thus vital and requires a solution. Anti-HPA1a antibodies are the most frequent cause of FNAIT in Caucasians. Its pathogenesis is similar to hemolytic disease of the newborn (HDN) due to anti-RhD antibodies, but is characterized by platelet destruction and is more often observed in the first pregnancy. In 75 % of these women, alloimmunization by HPA-1a antigens, however, occurs at delivery, which enables development of antibody-mediated immune suppression to prevent maternal immunization. As for HDN, the recurrence rate of FNAIT is high. For advancing diagnostic efforts and treatment, it is thereby crucial to understand the pathogenesis of FNAIT, including cellular immunity involvement. This review presents the current knowledge on FNAIT. Also described is a program for HPA-1a screening in identifying HPA-1a negative pregnant women at risk of immunization. This program is now performed at the Institute of Hematology and Transfusion Medicine in cooperation with the Department of Obstetrics and Gynecology of the Medical Centre of Postgraduate Education in Warsaw as well as the UiT The Arctic University of Norway.
Background Transfusion-related acute lung injury (TRALI) is currently one of the most common causes of transfusion-related major morbidity and death. Among the many TRALI mediators, leucocyte antibodies have been identified as important triggers of severe TRALI.
In this study, platelet counts were determined from the cord blood of consecutive 9142 newborns. Neonates with known autoimmune thrombocytopenia were not included. The platelet count < 100 x 10(9)/L was found in 64 newborns. In five of them, neonatal alloimmune thrombocytopenia (NAIT) was diagnosed. The overall incidence of neonatal thrombocytopenia was 0.7%, the incidence of NAIT was about 10 times less. Serological and clinical observations are summarized from 238 thrombocytopenic newborns (54 from the above group and 184 previously referred to serological investigations). All of the newborns were divided into two groups: NAIT (46 cases) and other thrombocytopenias (192 cases). Among platelet-specific antibodies in NAIT, 91.4% were anti-HPA-1a, the rest were anti-HPA-1b and anti-HPA-5b. In the majority of the cases, antibodies were detectable by the platelet suspension immunofluorescence test (PSIFT) and monoclonal antibody immobilization of platelet antigens (MAIPA) assay. In 19.6% cases, antibodies were detectable by MAIPA only. In 10.9% of these cases, antibodies were undetectable. Thrombocytopenia < 50 x 10(9)/L and hemorrhagic diathesis were more often observed in NAIT than in other thrombocytopenias, whereas associated disorders that could contribute to thrombocytopenia, here observed almost only in the latter group. We also report certain other observations, such as the presence of anti-HLA antibodies, a rise in the anti-HPA-1 a antibody titer after infection without pregnancy, and a higher incidence of petechiae in nonimmune thrombocytopenia as compared with the incidence of low platelet counts.
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